SEATTLE – Anthony James Davidson, age 6, is happy to show strangers his “zoop-zoop.”
That’s what the Seattle kindergartner calls the scar that zigzags across the top of his head, hidden by dark hair, permanent evidence that he was among the one in every 2,000 babies born with a birth defect that most parents don’t recognize – and can’t pronounce.
The boy, who goes by A.J., was diagnosed at 4 months with craniosynostosis – kray-nee-o-sin-os-TOE-sis – a condition in which one or more joints in a newborn’s skull fuse prematurely, preventing the brain from growing normally and leaving the baby with a misshapen head. He had surgery at 8 months, and then again last summer, when it appeared his skull was closing again.
A.J. says his scar is cool, but his mother, Shelby Davidson, 36, knows the fear and uncertainty that accompany the multi-hour surgery required to expand an infant’s skull.
For four years, she and Summer Ehmann, who lives in Colorado, have collaborated to send care packages – small boxes containing information and handmade hats and blankets – to thousands of families each year facing craniosynostosis.
“One of our main goals is to spread awareness,” said Davidson, a co-founder of the nonprofit group Cranio Care Bears. “Nine out of 10 of these parents haven’t heard of this.”
Davidson was lucky. A.J. was treated at Seattle Children’s Hospital, where the Craniofacial Center is among national leaders in treating craniosynostosis. Doctors there perform between 70 and 80 surgeries every month on babies newly diagnosed with the problem, said Dr. Michael Cunningham, the center’s medical director.
“It’s one of our most common diagnoses,” he said.
But most parents – and many pediatricians – don’t recognize the problem immediately, even though it’s present on the first day of life. “They think the head is molded from birth,” Cunningham said.
It’s only when the baby gets older and the skull doesn’t round out properly that parents start to worry.
An infant’s head is made up of bony plates interspersed with special joints, called sutures, between them. The sutures allow the child’s head to flex during the birth process, then they fuse slowly over time. Some sutures are solid by age 2 or 3, but the head usually isn’t completely fused until the teen years, doctors say.
With craniosynostosis, one or more sutures may be fused at birth. The type of disorder is determined by which suture fuses too early. A.J., for instance, was born with sagittal synostosis, the most common form of the disorder, accounting for about two-thirds of cases and affecting more boys than girls.
It occurs when the long suture that runs lengthwise across the top of the head fuses too soon, creating an elongated or boat-shaped skull.
“I knew something was wrong,” Shelby Davidson said. “It was like a ridge across the top of his head.”
Other forms of craniosynostosis can cause flattened or triangular-shaped foreheads, raised eye sockets or flattening at the back of the head. In some cases a baby’s eyes, ears or other features become asymmetrical because of the pressure of the growing brain on the hardened skull.
The cosmetic problem is a concern, but parents also worry that a hardened skull may stunt brain growth, causing intellectual and social problems. So far, evidence from the Infant Learning Project, a 5-year prospective study led by Matthew Speltz at Seattle Children’s, has found that kids with craniosynostosis may experience differences.
“They are delayed, but it’s not a very sizable delay,” said Speltz, who has followed children before and after surgery from birth through early elementary school, age 7 or 8.
Some cases of craniosynostosis are part of a larger syndrome caused by specific, well-known mutations that lead to disorders involving problems other than with the skull, Cunningham said. More common, however, are isolated cases of craniosynostosis, in which one suture is affected and there are no other problems.
The answer in nearly all cases is surgery. About half are performed endoscopically, in closed procedures, said Dr. Richard Hopper, surgical director of the Craniofacial Center. Kids who receive that treatment typically have to wear protective helmets for months.
The other half of the cases are like A.J.’s - open procedures that slice and expand the skull to make room for the growing brain.
“Is it scary? Oh, my gosh, yes,” said Shelby Davidson, who can’t forget the seven hours of waiting during A.J.’s first operation. “They’re basically taking his skull apart.”
Her baby came through the operation fine, with eyes swollen shut and evil-looking scars crisscrossing his head. But Davidson, who was alone for much of the procedure, said she vowed then to help others in the same situation.
“Right then, I knew what I was going to do,” she said.
Davidson connected with Summer Ehmann, whose son, Brentley, was born in 2010 with a rare form of craniosynostosis. Together, the two decided to send care packages to families across the country going through the same ordeal.
Both women’s stories are detailed in a forthcoming book by Kase Johnstun, a Utah State University writing teacher who was born with craniosynostosis in 1975 and details the medical history of the disorder and its impact on the lives of patients and families. The book is called “Beyond the Grip of Craniosynostosis: An Inside View of Life Touched by the Congenital Skull Deformity.”
Today, they mail about 100 boxes a month, half from Seattle, half from Ehmann’s home in Mead, Colorado. The boxes contain about $30 worth of comfort items, everything from a soft knit hat big enough to fit over a cranio baby’s expanded head to toothbrushes for frantic parents who often forget theirs.
Every box also contains a prayer chain of fabric loops on which other parents have written messages of hope and kindness. “The prayer chains make me cry,” Davidson said.
It’s a small gesture with a big impact. When Katie Long’s son, Camden, was diagnosed a year ago, the 31-year-old Bothell, Wash., mother says she was devastated.
She joined a Facebook group for other cranio parents and heard immediately from Shelby Davidson, who offered a care package – and to sit with Long during the surgery.
“It was so nice to have her there because she was so familiar with the process,” Long said. “Shelby is our angel.”
For Davidson, the project has created a kind of circle of caring.
When A.J. had his second surgery in July, three other mothers came to be with her. Six months later, strangers have to look hard to find the scar on A.J.’s otherwise normal-looking head.
His mother understands that support heals the scars that don’t show.
“It means so much,” she said. “We’ve had people say it’s like a hug from somebody who had been through it.”
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